Orbital Disease Research Articles

Value of Platelet-to-Lymphocyte and Monocyte-to-Lymphocyte Ratios in Differentiating Idiopathic Orbital Inflammation From Orbital Infectious Disease.

Monocyte-lymphocyte ratio (MLR) and platelet-lymphocyte ratio (PLR) are emerging biomarkers; they have not been widely utilized in orbital disease. This study investigated the role of these ratios in distinguishing orbital inflammation from infection. A retrospective review of medical records was conducted to identify adult patients who presented acutely to a single emergency department at an academic medical center and were diagnosed with serologically and biopsy-proven idiopathic orbital inflammation (IOI, n = 9), orbital cellulitis (OC, n = 14), or necrotizing fasciitis (NF, n = 12). MLR and PLR were calculated from the first blood draw on presentation to the emergency department. Statistical analysis was performed via the Mann-Whitney test with a dedicated computerized software package (GraphPad Prism, La Jolla, CA) with p values <0.05 considered statistically significant. Mean PLRs were 194.90 (SD = 118.01), 304.21 (SD = 341.39), and 203.38 (SD = 196.32) for IOI, NF, and OC, respectively. Mean MLRs were 0.40 (SD = 0.24), 1.74 (SD = 2.41), and 0.75 (SD = 0.40) for IOI, NF, and OC, respectively. MLR was significantly lower in the IOI group compared to the NF group (p = 0.018) and OC group (p = 0.020). Differences in MLR between OC and NF groups were not statistically significant (p = 0.43). While MLR showed no significant difference between different infectious etiologies, it was significantly higher in infectious processes than in IOI, distinguishing inflammation from infection. MLR could be a valuable addition to the diagnostic toolkit for triaging patients in the emergency department and initiating prompt, focused therapy.

The Neutrophil-Lymphocyte Ratio in Pediatric Preseptal and Orbital Infectious Cellulitis.

The neutrophil-lymphocyte ratio (NLR) is an important biomarker in a variety of systemic conditions, though its role in orbital disease remains unclear. We sought to investigate the utility of the NLR in distinguishing orbital cellulitis (OC) from preseptal cellulitis (PC) in a pediatric population. The earliest available NLRs of immunocompetent pediatric patients who presented to the emergency department with OC and PC were calculated from a single institution's database. Demographic and interventional data related to each encounter were also gathered. NLRs were compared via the Mann-Whitney test between OC and PC cases. Among patients with OC, the relationship between presenting NLR and requirement for surgical intervention and between presenting NLR and subperiosteal abscess was similarly examined. All statistical analyses were performed in GraphPad Prism 9 (Insight Partners, New York, NY, U.S.A.). Forty-three encounters of patients with OC and 23 encounters of patients with PC were identified. Median presenting NLR for the OC group was 4.18 (standard deviation: 5.44), whereas for the PC group, it was 1.96 (standard deviation: 2.38), representing a significant difference between the 2 groups (p < 0.001). The 26 patients with OC who required surgical intervention had significantly greater presenting NLR (median NLR: 6.53, standard deviation: 6.29) as compared to the 17 patients with OC who were managed medically (median NLR: 3.09, standard deviation: 2.37, p = 0.003). There was no statistically significant difference between 34 patients with OC who had a subperiosteal abscess (median NLR: 4.40, standard deviation: 5.91) and the 9 patients with OC who did not (median NLR: 3.84, standard deviation: 2.37, p = 0.401). An elevated NLR is a useful adjunct to the clinical examination in discerning OC from PC or in risk stratification for patients with OC who may need surgical treatment of their periocular infectious disease. In select cases, a reassuring NLR may allow the cost and radiation exposure associated with radiographic assessment to be avoided.

Thyroid-associated ophthalmopathy and ferroptosis: a review of pathological mechanisms and therapeutic strategies

Thyroid-associated ophthalmopathy (TAO) is an inflammatory orbital disease associated with autoimmune thyroid disorders. Owing to the ambiguous nature of the pathogenesis, contemporary pharmacological treatment strategies predominantly involve the use of glucocorticoids and immunosuppressants. However, the adverse effects associated with these agents in clinical practice necessitate further investigation into the disease’s pathogenesis and the identification of novel therapeutic targets and pharmacological interventions. Recent studies suggest that ferroptosis, a novel form of regulated cell death, may play a role in TAO pathogenesis. This review aims to explore the involvement of ferroptosis in TAO and evaluate its potential as a therapeutic target. Key topics include the epidemiology, clinical manifestations, and pathophysiology of TAO, along with the molecular mechanisms of ferroptosis. Evidence supporting ferroptosis in TAO and the therapeutic implications of targeting this pathway are also discussed, alongside challenges and future directions in this emerging research area.

Geographical distribution of human pythiosis in the USA.

For more than 100 years, the infections caused by Pythium insidiosum have been well documented in horses. However, recently, pythiosis was also described in several species, including dogs, cats, cattle, zoo captive animals, and humans. Human pythiosis is a life-threatening infection requiring an early diagnosis for a successful management. The clinical picture of pythiosis mirrors the clinical features of infections caused by other pathogens, including filamentous fungi, making its diagnosis challenging. This fact had led to several misdiagnosed cases involving filamentous fungal pathogens. The first pythiosis human case in the USA was reported in 1989. Soon after, various pythiosis cases of orbital disease in children and on the limbs of adults were sporadically diagnosed. To investigate the geographical distribution of human pythiosis in the USA, we constructed a density USA map using a recent report of pythiosis in animals highlighting the known human cases. Our data showed that P. insidiosum human infections in the USA are diagnosed in the same geographic areas where animal pythiosis occur. Physicians handling clinical cases like those so far reported can use the geographic distribution of pythiosis human cases in this study as a guide in their clinical diagnosis and in the early management of putative cases.IMPORTANCEThe relevance of the study not only resides in the epidemiological distribution of animal human pythiosis in the USA (our team's recent publication), but also shows physicians the areas where Pythium insidiosum thrive in endemic areas. Our manuscript shows to the medical and veterinary communities the current areas of human and animal pythiosis, an event that could have a direct impact on the epidemiology, diagnosis, and management of pythiosis.

Plasma cell type Castleman's disease of lacrimal gland: a case report and literature review

BackgroundOrbital Castleman's disease is a rare benign lymphoproliferative disorder of extranodal origin.Case presentationA 72-year-old man presented with bilateral upper eyelid swelling. Radiology revealed enlarged lacrimal glands in both eyes. Castleman's disease (plasma cell type) was confirmed by histo-immunochemical examinations after excision of the left orbital mass. No extraorbital involvement was found in systemic evaluation. Postoperatively, corticosteroid therapy was given. After 1 year of follow-up, there was no recurrence in the left orbit, and the swelling of the right eyelid was improved.ConclusionsHere, we report a case of orbital Castleman's disease involving the lacrimal gland bilaterally and summarize previously reported cases. For bilateral orbital masses, the possibility of Castleman's disease should be considered. Before the diagnosis, it is necessary to fully distinguish from other diseases such as IgG4-related eye diseases.

Orbital Inflammatory Disease as a Presenting Symptom of Generalized Lipodystrophy in a Young Female.

We report the first case of generalized lipodystrophy with orbital inflammatory symptoms. A 6-year-old female with hypothyroidism who developed hepatosplenomegaly, lymphadenopathy, and progressive loss of subcutaneous fat. Following flu vaccination, she developed orbital inflammatory symptoms. Imaging of the orbits demonstrated a paucity of retrobulbar fat and fat stranding. Systemic workup revealed insulin resistance and hepatosteatosis, consistent with generalized lipodystrophy. We discuss the typical history and examination findings in generalized lipodystrophy and review the etiology, treatment options, and outcomes.

Performance of ChatGPT in Ophthalmic Registration and Clinical Diagnosis: Cross-Sectional Study.

Artificial intelligence (AI) chatbots such as ChatGPT are expected to impact vision health care significantly. Their potential to optimize the consultation process and diagnostic capabilities across range of ophthalmic subspecialties have yet to be fully explored. This study aims to investigate the performance of AI chatbots in recommending ophthalmic outpatient registration and diagnosing eye diseases within clinical case profiles. This cross-sectional study used clinical cases from Chinese Standardized Resident Training-Ophthalmology (2nd Edition). For each case, 2 profiles were created: patient with history (Hx) and patient with history and examination (Hx+Ex). These profiles served as independent queries for GPT-3.5 and GPT-4.0 (accessed from March 5 to 18, 2024). Similarly, 3 ophthalmic residents were posed the same profiles in a questionnaire format. The accuracy of recommending ophthalmic subspecialty registration was primarily evaluated using Hx profiles. The accuracy of the top-ranked diagnosis and the accuracy of the diagnosis within the top 3 suggestions (do-not-miss diagnosis) were assessed using Hx+Ex profiles. The gold standard for judgment was the published, official diagnosis. Characteristics of incorrect diagnoses by ChatGPT were also analyzed. A total of 208 clinical profiles from 12 ophthalmic subspecialties were analyzed (104 Hx and 104 Hx+Ex profiles). For Hx profiles, GPT-3.5, GPT-4.0, and residents showed comparable accuracy in registration suggestions (66/104, 63.5%; 81/104, 77.9%; and 72/104, 69.2%, respectively; P=.07), with ocular trauma, retinal diseases, and strabismus and amblyopia achieving the top 3 accuracies. For Hx+Ex profiles, both GPT-4.0 and residents demonstrated higher diagnostic accuracy than GPT-3.5 (62/104, 59.6% and 63/104, 60.6% vs 41/104, 39.4%; P=.003 and P=.001, respectively). Accuracy for do-not-miss diagnoses also improved (79/104, 76% and 68/104, 65.4% vs 51/104, 49%; P<.001 and P=.02, respectively). The highest diagnostic accuracies were observed in glaucoma; lens diseases; and eyelid, lacrimal, and orbital diseases. GPT-4.0 recorded fewer incorrect top-3 diagnoses (25/42, 60% vs 53/63, 84%; P=.005) and more partially correct diagnoses (21/42, 50% vs 7/63 11%; P<.001) than GPT-3.5, while GPT-3.5 had more completely incorrect (27/63, 43% vs 7/42, 17%; P=.005) and less precise diagnoses (22/63, 35% vs 5/42, 12%; P=.009). GPT-3.5 and GPT-4.0 showed intermediate performance in recommending ophthalmic subspecialties for registration. While GPT-3.5 underperformed, GPT-4.0 approached and numerically surpassed residents in differential diagnosis. AI chatbots show promise in facilitating ophthalmic patient registration. However, their integration into diagnostic decision-making requires more validation.

First person – Yujiao Wang

ABSTRACT First Person is a series of interviews with the first authors of a selection of papers published in Disease Models &amp; Mechanisms, helping researchers promote themselves alongside their papers. Yujiao Wang is first author on ‘ A high-fat plus high-sucrose diet induces age-related macular degeneration in an experimental rabbit model’, published in DMM. Yujiao is a Lecturer and Ophthalmologist in the lab of Danian Chen at West China Hospital, Sichuan University, investigating Animal models of ocular disease (retinal disease and orbital disease).

Expert consensus on the diagnosis and treatment of immunoglobulin-G4 related ophthalmic disease (2024 edition)

Immunoglobulin-G4 related ophthalmic disease (IgG4-ROD) is an immune-mediated fibroin flammatory condition characterized by ocular mass lesions and elevated plasma IgG4 level. Since all the ocular tissues can be involved, and it can be accompanied by multiple organ and systemic diseases, the diagnosis and treatment require multidisciplinary collaboration. The Oculoplastic and Orbital Disease Group of Chinese Ophthalmological Society of Chinese Medical Association and the Chinese Rheumatology Association convened experts in ophthalmology, and rheumatology and immunology, and formulated "Expert consensus on the diagnosis and treatment of immunoglobulin-G4 related ophthalmic disease (2024 edition)" for the diagnosis and treatment of IgG4-ROD based on domestic and international studies and experience. The consensus mainly standardized the diagnostic criteria of IgG4-ROD from the aspects of ocular imaging, serum IgG4 level and ocular histopathology, and recommended the methods and indications of drug therapy (glucocorticoids, immunosuppressants and biologics) and surgical treatment.

Advancements in equine ophthalmic imaging enhance understanding of ocular and orbital anatomy and disease in standing sedated horses.

To review data on the advances in equine ophthalmic imaging that have been made during the past 5 years and highlight advantages of using multiple imaging modalities to improve clinical observational skills and improve diagnostic accuracy. A literature review from 2019 through 2024 of equine ophthalmic digital photography, fundus photography, ocular and orbital ultrasonography (US), ultrasound biomicroscopy (UBM), confocal microscopy (CM), spectral domain optical coherence tomography (SD-OCT), radiography, CT, and MRI. Digital photography remains the cornerstone of equine ophthalmic imaging for documenting examination findings, sharing information with colleagues, and consulting with specialists. Digital images also allow for in-depth postexamination review and evaluation, often revealing subtleties that may have otherwise gone undetected during the ophthalmic examination. Advanced imaging modalities are being used more frequently in equine ophthalmology, especially those that can be used with the horses standing under sedation, including US, UBM, SD-OCT, CM, and CT. Advances in equine ophthalmic imaging have led to many new clinical discoveries and to an increase in our knowledge of ocular anatomy and diseases in the horse. Many of these advanced diagnostic imaging modalities, such as MRI, CT, SD-OCT, and CM, are cost prohibitive and require substantial operator training to ensure proficiency. However, their availability in tertiary referral centers, such as veterinary teaching hospitals and large equine clinics/practices, is becoming more widespread. Advanced equine ophthalmic imaging data contributes substantially to our general understanding of clinical and applied anatomy and improves our understanding of the underlying pathogenesis associated with specific diseases.

Probable IgG4-related Orbital Disease Masked by Exuberant Ocular Surface Pseudoepitheliomatous Hyperplasia

A 40-year-old woman presented with a mass in her OS for 2 years. Examination revealed a large conjunctival lesion on the nasal bulbar conjunctiva OS and a small upper tarsal conjunctival lesion in the OD. Biopsy OD revealed inflammatory granulation tissue, and OS revealed pseudoepitheliomatous hyperplasia with granulation tissue. The lesion responded well to steroids. She was lost to follow-up and returned with an enlarged lesion with orbital extension and corneal perforation. Orbital exenteration was performed. The entire conjunctiva showed extensive epithelial downgrowth and mononuclear round cell infiltrates, predominantly plasma cells admixed with neutrophils suggestive of aggressive pseudoepitheliomatous hyperplasia. Orbital tissue showed ill-defined granuloma formation with acute on chronic inflammation and plasma cells. The tissue IgG4/IgG ratio was 34%, suggestive of probable IgG4-related orbital disease. Based on this case, we conclude that IgG4-related orbital disease can rarely cause conjunctival and scleral inflammation with secondary pseudoepitheliomatous hyperplasia-like changes.

Targeted orbital intervention in the management of sino-orbital mucormycosis cases.

Many countries from South-East Asia reported an epidemic of sino-orbital mucormycosis (SOM), otherwise a rare disease, during the coronavirus disease 2019 pandemic. SOM, a potentially fatal disease, is typically treated with orbital exenteration and systemic antifungals after metabolic stabilization. There is no clear evidence of survival benefit of exenteration in the literature, and thus, there have been attempts at globe conserving treatments like orbital infusion after limited debridement and intraorbital injections with Amphotericin B (IOAB). We conducted a prospective comparative interventional study at a tertiary eye care hospital to evaluate treatment outcomes with the use of adjunctive IOAB in cases of SOM with mild to moderate orbital disease. Thirty-six patients of SOM with mild to moderate orbital disease were recruited in the study. In the intervention group, 23/26 (885%) eyes had stable orbital disease at the end of treatment (4-6 weeks). No deterioration in visual acuity was noted as a result of treatment. In 8/26 (30.77%) patients, inflammation was noted as a side effect of IOAB requiring temporary discontinuation of injections. The mean follow-up for cases was 14.2 months (range 12-15 months). 1/23 (4.35%) patients had relapse of orbital disease at 3 months. Twenty-one patients are alive on last follow-up. Of the patients who refused treatment (controls), 2/9 (22.22%) patients relapsed. One of these patients with relapse underwent exenteration, while the other was managed with IOAB. At a follow-up of 14 months (range 12-15 months), eight patients are alive. On evaluating the ocular parameters in salvaged eyes, improvement in extraocular movements was noted in 75-80% cases. The degree of proptosis and resistance to retropulsion did not change significantly. In the current study, an improvement in the globe salvage rates was noted in cases of SOM with mild to moderate orbital disease treated with adjunctive IOAB as compared to controls at a mean follow-up of 14 months, although it did not achieve statistical significance. The study supports the inclusion of IOAB in routine management of mild to moderate orbital disease.

Atypical presentation of sphenoid wing meningioma mimicking idiopathic orbital inflammation

Sphenoid wing meningiomas (SWMs) are slow-growing tumors that commonly present with proptosis and progressive visual deterioration. Due to the proximity of the sphenoid bone to key structures in the orbit, it may occasionally be misdiagnosed as other inflammatory and other neoplastic orbital diseases. This case report highlights an atypical presentation of SWM that mimics idiopathic inflammatory orbital disease, underscoring the importance of differential diagnosis.

Analysis of anthropometric measurements taken from the periocular region in healthy young individuals with different somatotypes.

Anthropometric parameters related with the periocular region (PR) have gained great importance with the optical technology used in the treatment of eye defects. They have also become important parameters that determine the limits of treatment in aesthetic surgery procedures in PR and the treatment of orbital diseases. The aim of this study was to examine the relationship of PR measurements with somatotype. Somatotypes were determined by using the Heath-Carter method. Ten indirect anthropometric measurements, namely nasal root (mf-mf), outer chantal distance (ex-ex), inner chantal distance (en-en), interpupillary distance (p-p), right and left palpebral fissure width (ex-en), palpebral fissure height (ps-pi), and orbital height (os-oi), were taken from PR with Image J program. Kruskal-Wallis H test was used to compare data with each other. Post-hoc Mann-Whitney U test was used to find out which group caused statistically significant differences according to the results of the Kruskal-Wallis H test. A statistically significant difference was found between some indirect anthropometric measurements taken from PR and somatotypes (P < 0,05). This difference was found to result from central group somatotype, in which no somatotype component has different effects from another one, for both genders. In almost all of the indirect anthropometric measurements, it was found that balanced ectomorph somatotypes reached the highest median values, while central group somatotypes had the lowest median values. As a result of this study, somatotype affects the appearance of PR. It will be possible to obtain more accurate and reliable results in aesthetic surgery interventions and identification studies. Our study is the first and a pioneer in its field in the literature. It will inspire and guide future researchers, surgeons, anthropologists, and forensic doctors who will work in this field.

Central Retinal Artery Occlusion Following Intralesional Triamcinolone Injection for IgG4-related Orbital Disease.

The risk of blindness associated with periocular and facial injections is well documented. Previous cases describing vision loss following a recent periocular or facial injection have emphasized the importance of facial "danger zones." To date, the literature suggests that nearly half of all cases of central retinal artery occlusion in the setting of a recent periocular or facial injection involve an injection in or around the nose. Here, the authors report the second known case of central retinal artery occlusion following a triamcinolone injection to the lacrimal gland. A 30-year-old female with a diagnosis of IgG4-related disease underwent lacrimal gland debulking with an intralesional steroid injection. She noted OD vision loss immediately after surgery, with posterior segment examination demonstrating retinal whitening with a cherry-red spot and intra-arterial yellow-white plaques. This case serves as a reminder to clinicians regarding the risk of iatrogenic embolism following triamcinolone injections around the face with special attention drawn to the lacrimal gland.

Evaluation of the specificity of trigeminal nerve enlargement in the diagnosis of IgG4-related ophthalmic disease.

Enlargement of the trigeminal nerve is observed in 20-53% of patients with IgG4-related ophthalmic disease (IgG4-ROD) and is known to be a useful finding for the diagnosis of IgG4-ROD. On the other hand, enlargement of the trigeminal nerve has also been found at a certain frequency in orbital lymphoproliferative diseases other than IgG4-ROD. Therefore, we here re-evaluated the specificity of trigeminal nerve enlargement in the diagnosis of IgG4-ROD. Retrospective, comparative study. A total of 149 consecutive cases of IgG4-ROD diagnosed at the Department of Ophthalmology, Tokyo Medical University Hospital were studied. As controls, 218 cases of orbital lymphoma, 13 cases of reactive lymphoid hyperplasia (RLH), and 117 cases of benign orbital tumors other than lymphoproliferative diseases were included. Enlargement of the trigeminal nerve (infraorbital or supraorbital nerve) in IgG4-ROD and all the control cases was evaluated on MRI or CT coronal images. Enlargement of the trigeminal nerve was observed in 35 of the 149 cases (23.5%) of IgG4-ROD and in 7 of the 218 cases (3.2%) of lymphoma, with a significantly highly frequency in IgG4-ROD (P < .0001). No cases of trigeminal nerve enlargement were observed in the cases of RLH or benign orbital tumors. The sensitivity and the specificity of trigeminal nerve enlargement in the diagnosis of IgG4-ROD were 23.5% and 96.8%, respectively. Additionally, enlargement of the trigeminal nerve was significantly more common in men than in women (P < .028). The present study indicates that trigeminal nerve enlargement is a characteristic imaging finding and has diagnostic value for IgG4-ROD.

Differentiation of bacterial orbital cellulitis and diffuse non-specific orbital inflammation on magnetic resonance imaging.

To examine the radiological differences between bacterial orbital cellulitis (OC) and diffuse non-specific orbital inflammation (DNSOI) on magnetic resonance imaging (MRI). Retrospective study of patients with OC and DNSOI with an MRI orbital scan. Localised orbital inflammation (e.g., idiopathic dacryoadenitis and myositis), quiescent orbital inflammation and pre-septal cellulitis were excluded. Thirty-two patients presenting between 2008 and 2023, including twenty-one OC patients (mean age: 42.5 ± 24.9 years old, male: 6), and eleven DNSOI patients (mean age: 52.3 ± 17.8 years old, male: 16). Both OC and DNSOI demonstrate orbital fat contrast-enhancement. However, whilst OC demonstrated a hyperintense T2 signal (P < 0.001), variable signal was observed in DNSOI, with a hypointense T2 signal more suggestive of DNSOI (P = 0.012). When the lacrimal glands were involved, indistinct margins were more likely in OC (P < 0.001), whilst gross enlargement and contrast-enhancement was observed in DNSOI (P = 0.032 and 0.017, respectively). Peripheral contrast-enhancement of the extraocular muscle (EOM) (P = 0.002) was more common in OC, whilst DNSOI demonstrated variable contrast-enhancement throughout the affected EOM (P < 0.001). The presence of contralateral abnormalities, such as lacrimal gland enlargement and EOM involvement, are more suggestive of DNSOI. Several MRI features, beyond overt sinogenic disease, may help to differentiate OC from DNSOI, including the orbital fat signal intensity, EOM and/or lacrimal gland involvement, and contralateral orbital abnormalities. However, these features may not be specific, and thus highlights the ongoing radiological dilemma clinicians are faced when tasked with differentiating between infectious and non-infectious orbital inflammatory disease.

Non-infectious hypertrophic pachymeningitis associated with orbital inflammatory disease: a pooled analysis.

To describe four cases of non-infectious hypertrophic pachymeningitis (HP)-associated with orbital inflammatory disease (OID). This study summarises the clinico-radiological features, outcomes, and management of HP-associated OID. Retrospective case-series of patients with radiological evidence of HP and OID. Comprehensive literature review of all published English-language non-infectious causes of HP-associated OID. Reference lists were screened for inclusion of relevant articles. Thirty-seven cases of HP-associated OID (Mean age: 49.2 ± 17.4 years old; Male: 15) were identified, including four cases from our institution. Aetiologies included ANCA-associated vasculitis (12/37), non-specific/idiopathic (11/37), IgG4/multifocal fibrosclerosis (11/37), neurosarcoidosis (1/37), inflammatory myofibroblastic tumour (1/37), and giant cell arteritis (1/37). Orbital pain, headache, visual deterioration, and cranial nerve palsies were common clinical presentations. Both "focal" and "diffuse" HP were observed, with the most common sign of orbital involvement being an inflammatory orbital mass, typically with orbital apex involvement. Orbital myositis and dacryoadenitis were less common. The cavernous sinus was the most common site of extra-orbital inflammation. There was no single differentiating specific radiological feature between non-specific and specific forms of HP-associated OID. The clinico-radiological manifestations of HP-associated OID differ from those described in isolated HP or OID. There is no single specific radiological marker differentiating non-specific/idiopathic disease from secondary causes; however, the co-existence of HP in OID should prompt suspicion of an underlying cause. The disease may be refractory or resistant to initial treatment, although guidelines surrounding its management and the long-term prognosis remain to be determined.

Extraocular muscle Diffusion Weighted Imaging as a quantitative metric of posterior orbital involvement in thyroid associated orbitopathy

ObjectivesThe clinical activity score (CAS) and European severity scale (ESS) are established clinical tools to assess thyroid eye disease (TED) but are limited in terms of subjectivity and their reliability in non-Caucasian individuals, and can underestimate significant disease in the posterior orbit. Preliminary data from pilot studies have shown that diffusion-weighted imaging (DWI) using extraocular muscle (EOM) apparent diffusion coefficient (ADC) measurements may provide complementary information in TED. This study expands on previous research to assess for correlations between clinical scores and EOM-ADCs in stratifying disease activity and severity in a large patient cohort from an ethnically diverse population.MethodsA retrospective review of TED clinics between 2011 and 2021 identified 96 patients with a documented CAS and ESS and an orbital MRI that included DWI. From regions of interest manually placed on EOM bellies, the highest ADC was computed for each patient and analysed for correlations and associations with CAS and ESS using Spearman Rank correlation and Mann–Whitney U tests, and any potential discriminatory cut-offs using Receiver Operator Curve analyses. A p-value < 0.05 indicated statistical significance.ResultsEOM-ADCs showed a positive association with CAS (p ≤ 0.001). EOM-ADCs were higher in sight-threatening compared to mild disease (p ≤ 0.01). A cut-off of 995 mm2/s achieved AUC = 0.7744, equating to 77% sensitivity and 67% specificity for discrimination between mild-moderate and sight-threatening disease.ConclusionEOM-ADCs correlate with higher scores of disease severity and activity in TED. Besides providing quantitative data to support clinical tools, EOM-ADC cut-offs may identify patients at risk of developing sight-threatening diseases.Critical relevance statementThis study critically evaluates the limitations of conventional clinical assessment tools for TED and demonstrates the utility of DWI scans with ADC measurements in identifying active disease, offering valuable insights to advance clinical radiology practice.Key PointsConventional tools for TED assessment have subjective limitations.ADCs from non-echoplanar diffusion-weighted imaging correlate with clinical activity.Non-echoplanar diffusion-weighted imaging offers quantitative assessment to aid clinical practice reliability.Graphical

Prediction models of intravenous glucocorticoids therapy response in thyroid eye disease.

Thyroid eye disease (TED) is an autoimmune orbital disease, with intravenous glucocorticoid (IVGC) therapy as the first-line treatment. Due to uncertain response rates and possible side effects, various prediction models have been developed to predict IVGC therapy outcomes. A thorough search was conducted in PubMed, Embase, and Web of Science databases. Data extraction included publication details, prediction model content, and performance. Statistical analysis was performed using R software, including heterogeneity evaluation, publication bias, subgroup analysis, and sensitivity analysis. Forest plots were utilized for result visualization. Of the 12 eligible studies, 47 prediction models were extracted. All included studies exhibited a low-to-moderate risk of bias. The pooled area under the receiver operating characteristic curve (AUC) and the combined sensitivity and specificity for the models were 0.81, 0.75, and 0.79, respectively. In view of heterogeneity, multiple meta-regression and subgroup analysis were conducted, which showed that marker and modeling types may be the possible causes of heterogeneity (P < 0.001). Notably, imaging metrics alone (AUC = 0.81) or clinical characteristics combined with other markers (AUC = 0.87), incorporating with multivariate regression (AUC = 0.84) or radiomics analysis (AUC = 0.91), yielded robust and reliable prediction outcomes. This meta-analysis comprehensively reviews the predictive models for IVGC therapy response in TED. It underscores that integrating clinical characteristics with laboratory or imaging indicators and employing advanced techniques like multivariate regression or radiomics analysis significantly enhance the efficacy of prediction. Our research findings offer valuable insights that can guide future studies on prediction models for IVGC therapy in TED.